Temporal bone cancer, a relatively rare disease, often presents in a subtle manner, which may delay diagnosis. It should be suspected in any case of persistent otitis media or otitis externa that fails to improve with adequate treatment. This article discusses physical and diagnostic imaging indications and epidemiology and histologic distribution of these tumors. Staging systems, patient selection criteria are presented as well as operative techniques and follow-up treatment. Despite advances in operative technique and postoperative care, long-term survival remains poor.